Kartagener's syndrome: a case report

Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disease, and is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus. This paper reports the case of a 27-year-old female presenting with dyspnea on medium exertion, accompanied by chronic cough, non-productive or with clear expectoration. She had recurrent pneumonia until 15 years of age and underwent a lobectomy in the lower lobe of the left lung, probably due to bronchiectasis. Chest computed tomography showed situs inversus totalis, signs of previous surgical manipulation, and mild bronchial thickening. Computed tomography of the paranasal sinuses showed signs of chronic sinusitis due to a probable ciliary kinesis disorder. These finding suggest the diagnosis of Kartagener's syndrome. The prognosis reveals a slow rate of decline in lung function. However, repeated or chronic infections can negatively influence the quality of life of these patients.

Situs inversus totalis asociado a síndrome de preexcitación ventricular: reporte de un caso / Situs inversus totalis and pre excitation syndrome

Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.

Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review / Púrpura trombocitopênica idiopática em paciente com situs inversus totalis: relato de caso e revisão da literatura

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.

Tetralogy of Fallot in a Case of Dextrocardia with Situs Solitus.

Dextrocardia associated with situs solitus (so called ‘dextroversion’) is a cardiac positional anomaly in which the heart is located in the right hemithorax with its base-toapex axis directed to the right and caudad along with normal anatomical position of other intrathoracic and abdominal viscera. The malposition is intrinsic to the heart and not caused by extracardiac abnormalities. Dextroversion is the second most common type of dextrocardia. In dextroversion, there is a 90% incidence of additional cardiac malformations, including anomalous pulmonary venous return, Tetralogy of Fallot, septal defects, pulmonic stenosis, coarctation of the aorta, and corrected TGA.Here we report a rare case of Tetralogy of Fallot in a 17 year old male with dextroversion.

Removal of Choledocholith by Endoscopic Retrograde Cholangiopancreatography in a Situs Invsersus Patient / 대한소화기학회지

Situs inversus is an extremely rare autosomal recessive disease with left-right inversion of internal organs. It carries technical difficulties in diagnostic or therapeutic procedures. There have been a few case reports on stone extraction by ERCP in situs inversus patients. ERCP techniques in situs inversus can be classified into conventional method and mirror image method. In mirror image method, the procedure is performed with the patient in the right lateral decubitus position and the endoscopist on the patient's left side. Until now, there is no consensus about which method is better. Herein, we report an unusual case of choledocholithiasis in a patient with situs inversus who underwent ERCP for stone extraction by both conventional method and mirror image method.

Successful Stone Removal by Endoscopic Retrograde Cholangiopancreatography in Situs Inversus Totalis with Billroth-II Gastrectomy

No abstract available.

How standard transesophageal echocardiography views change with dextrocardia.

Dextrocardia with situs inversus is a rare condition. Situs inversus with dextrocardia is also called as "situs inversus totalis". Transesophageal echocardiography (TEE) views in dextrocardia patient are not discussed in the literature. The cardiac position and the cardiac chambers are mirror image of the normal anatomy. Because of this positional change, certain TEE probe and multiplane angle manipulations are required to obtain the recommended views.

Hepatectomia por linfangioma em situs inversus totalis : relato de caso / Hepatectomy of lymphangioma in situs inversus totalis : case report

We present a case of hepatectomy in a patient with liver's lymphangioma associated with Situs Inversus Totalis. The first case of medical literature.

Derivação gastrojejunal com anel de contenção no tratamento de obesidade mórbida em paciente com situs inversus totalis / The gastric bypass banding for morbid obesity in a patient with situs inversus

The gastric bypass is a good option in the therapy of morbid obesity. Nevertheless, it must be considered the rare condition as occurred in a patient with previous abdominal surgery with Situs Inversus Totalis. A 24 year-old male patient with body mass index of 40 Kg/ m², multiple dietary failures, and arterial hypertension as co-morbidities, with a anterior paramedial right incision due to a previous appendicectomy (8 years ago).With a indication for bariatric surgery, was performed Roux-en-Y gastric bypass by laparoscopic procedure, with previous planning of Situs Inversus Totalis.

Laparoscopic Total Mesorectal Excision in a Rectal Cancer Patient with Situs Inversus Totalis

Situs inversus totalis is a rare anomaly in which the abdominal and thoracic cavity structures are opposite their usual positions. A 41-yr-old woman, who had an ulcerating cancer on the rectum, was found as a case of situs inversus totalis. We present an overview of the operative technique for the first documented laparoscopic total mesorectal excision of a rectal cancer in the patient with situs inversus totalis. Careful consideration of the mirror-image anatomy permitted a safe operation using techniques not otherwise different from those used for the general population. Therefore, curative laparoscopic surgery for rectal cancer in this patient is feasible and safe.

Consideraciones técnicas durante la colecistectomía laparoscópica en paciente con situs inversus totalis / Technical considerations during laparoscopic cholecystectomy in a patient with situs inversus totalis

Introducción: La colecistectomía laparoscópica es uno de los procedimientos quirúrgicos más efectuados por el cirujano general. Esto ha permitido establecer una estrategia quirúrgica bien definida para su ejecución; sin embargo, ciertos factores pueden llevar a modificar la técnica. Los objetivos de este informe son describir y analizar las variaciones de la técnica quirúrgica y los resultados obtenidos con la colecistectomía laparoscópica en una paciente con situs inversus totalis. Caso clínico: Mujer de 70 años de edad con diagnósticos de colecistopatía crónica litiásica y situs inversus totalis. Detallamos los cambios en la técnica quirúrgica y describimos la variación en la distribución anatómica de los órganos intraabdominales observada durante el procedimiento quirúrgico. La paciente evolucionó satisfactoriamente y refirió alto grado de satisfacción con el resultado quirúrgico. Conclusiones: La variación en la distribución anatómica de los órganos intraabdominales, así como la alteración en la localización de la arteria cística y los cambios en la ubicación de los puertos de trabajo, nos indujeron a modificar la técnica de la colecistectomía laparoscópica, lo cual no alteró el resultado quirúrgico ni la evolución posoperatoria.

BACKGROUND: Laparoscopic cholecystectomy is one of the most frequently performed surgical procedures by the general surgeon. This situation allows determining a defined surgical strategy in most patients. However, some factors may induce modification by the surgical team. Our objectives were to describe and analyze the variation of the surgical technique used during laparoscopic cholecystectomy in a female patient with situs inversus totalis. CLINICAL CASE: We report the case of a 70-year-old female with a diagnosis of chronic gallstone cholecystitis and situs inversus totalis. Modifications in the surgical technique are detailed, and anatomic variations of the intraabdominal organs are described. Postoperative evolution was satisfactory and the patient was highly satisfied with the surgical outcome. There was no associated morbidity. CONCLUSIONS: Variations in intraabdominal anatomy, alteration of cystic artery location and changes in position of the abdominal ports prompted us to modify the standard surgical technique for laparoscopic cholecystectomy. However, these factors did not alter results or the patient's postoperative evolution.

Dextrocardia: reporte de caso y revisión de literatura / Dextrocardia: case report and literature report

La dextrocardia es una malformación que ocurre en el período embrionario y se caracteriza por el desplazamiento de la masa y del eje cardíaco principal (la base del ápex) hacia la derecha del tórax. Se reporta el caso de una paciente de diez años de edad quien consultó inicialmente por un cuadro de faringoamigdalitis a repetición y elevación de antiestreptolisinas. La exploración física y estudios de gabinete demostraron la presencia de dextrocardia sin situs inversus. El tratamiento de la infección estreptocócica fue efectivo y la paciente evolucionó satisfactoriamente sin complicaciones asociadas a la malformación, continuando su seguimiento por consulta externa...

Obstruction of the duodenum by a preduodenal portal vein in situs inversus

Mirror image transposition of abdominal and thoracic viscera is termed situs inversus. Duodenal obstruction in situs inversus is rare. A preduodenal portal vein, though not uncommon in situs inversus, rarely causes duodenal obstruction. Where obstruction by a preduodenal portal vein is diagnosed, a duodeno-duodenostomy is the recommended treatment. A duodenal diaphragm and other more common causes of duodenal obstruction should also be excluded in these patients.

Revascularização do miocárdio em paciente com situs inversus totalis / Myocardial revascularization in a patient with situs inversus totalis

Relatamos o caso de um paciente com situs inversus totalis associado a coronariopatia obstrutiva em artérias descendente anterior e posterior, coronária direita, primeiro ramo diagonal e ramo marginal esquerdo, quadro condizente com a intervenção de revascularização do miocárdio. Esse procedimento não é freqüente na literatura médica, sendo encontrado apenas um relato na literatura brasileira. A revascularização do miocárdio foi realizada com a artéria mamária interna direita para a artéria descendente anterior, e uma ponte de safena para coronária direita, marginal esquerda, primeiro ramo diagonal e descendente posterior. A cirurgia foi realizada com o auxílio de circulação extracorpórea.

We report the case of a patient with dextrocardia and situs inversus totalis associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal branch. The patient underwent coronary artery bypass grafting surgery. This surgery has been rarely reported in literature and we found only one similar case in the national medical literature. The myocardial revascularization was carried out with the right mammary artery for the anterior descending artery. The saphenous vein anastomosed the aorta to the right coronary artery, left marginal branch, fist diagonal branch and posterior descending artery. The surgery was performed with extracorporeal circulation.

Malrotation of the intestine and preduodenal portal vein associated with abdominal situs inversus: a case report

Abdominal situs inversus is a rare condition usually associated with malformations of asymmetric organs such as the heart, liver, spleen and malrotation of the intestines. A case of abdominal situs inversus with intestinal malrotation and preduodenal portal vein is reported. Patient underwent prophylactic Ladd's procedure and preduodenal portal vein was left undisturbed during surgery. This case highlights the importance of rigorous investigation of anatomic features prior to surgery in a patient with heterotaxia. The authors advocate radiological investigation of patients with heterotaxia and prophylactic Ladd's procedure in those with intestinal malrotation.

Splenic abscess in a boy with isolated levocardia.

Levocardia with situs inversus is a rare condition. We present the case of a fourteen-year-old boy with congenital cyanotic heart disease, isolated levocardia and splenic abscess. It is the first report of both these conditions occurring in the same person.